ArticleThe Journal of clinical investigation1998
Highly restricted human T cell repertoire in peripheral blood and tissue-infiltrating lymphocytes in Omenn's syndrome.
Article in The Journal of clinical investigation, 1998. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 31 papers.
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Who cites it
31 citing papers in PubMed, 88 citations in OpenAlex.
- IgE Production Beyond Classical T-Cell Help.Immune network · 2026Review
- Autoimmunity in Primary Immunodeficiencies (PID).Clinical reviews in allergy & immunology · 2023Review
- The recombinase activating genes: architects of immune diversity during lymphocyte development.Frontiers in immunology · 2023Review
- Breast cancer is marked by specific, Public T-cell receptor CDR3 regions shared by mice and humans.PLoS computational biology · 2021Article
- Diagnostic and Predictive Contribution of Autoantibodies Screening in a Large Series of Patients With Primary Immunodeficiencies.Frontiers in immunology · 2021Article
- Predicting the Occurrence of Variants in RAG1 and RAG2.Journal of clinical immunology · 2019Article
- Approaches to patients with variants in RAG genes: from diagnosis to timely treatment.Expert review of clinical immunology · 2019Review
- Recent advances in understanding RAG deficiencies.F1000Research · 2019Review
- RAG Deficiency: Two Genes, Many Diseases.Journal of clinical immunology · 2018Review
- RAGs and BUGS: An alliance for autoimmunity.Gut microbes · 2016Article
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- Human RAG mutations: biochemistry and clinical implications.Nature reviews. Immunology · 2016Review
- Intestinal microbiota sustains inflammation and autoimmunity induced by hypomorphic RAG defects.The Journal of experimental medicine · 2016Article
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- Secondary immunologic consequences in chromosome 22q11.2 deletion syndrome (DiGeorge syndrome/velocardiofacial syndrome).Clinical immunology (Orlando, Fla.) · 2010Article
- Homeostatic expansion of autoreactive immunoglobulin-secreting cells in the Rag2 mouse model of Omenn syndrome.The Journal of experimental medicine · 2010Article
- Molecular analysis of T-B-NK+ severe combined immunodeficiency and Omenn syndrome cases in Saudi Arabia.BMC medical genetics · 2009Article
- A RAG1 mutation found in Omenn syndrome causes coding flank hypersensitivity: a novel mechanism for antigen receptor repertoire restriction.Journal of immunology (Baltimore, Md. : 1950) · 2008Article
- Lack of iNKT cells in patients with combined immune deficiency due to hypomorphic RAG mutations.Blood · 2008Article
Corrections and comments
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Authors and funding
7 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Omenn's syndrome is an inherited human combined immunodeficiency condition characterized by the presence of a large population of activated and tissue-infiltrating T cells. Analysis of the TCRB repertoire revealed a highly restricted TCRBV usage in three patients. More strikingly, T cell clones from the three patients expressed TCRB chains with VDJ junction similarities, suggesting a common antigenic specificity. Analysis of the TCRA repertoire in one patient also revealed a restricted TCRAV usage. Finally, analysis of the TCRBV repertoire of tissue-infiltrating T cells in one patient suggested nonrandom tissue migration. These results suggest that the oligoclonal expansion of T cells observed in Omenn's syndrome could be the consequence of autoimmune proliferation generated by a profound defect in lymphocyte development.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.